What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

We report a case of common bile duct (CBD) cancer, successfully managed with pancreaticoduodenectomy, in a patient with isolated levocardia, malrotation, and situs ambiguous (without splenic anomalies). A 59-year-old male patient was referred to Chung-Ang University Hospital with epigastric pain and jaundice. CT and MRI revealed distal CBD cancer without significant lymphadenopathy. Multiple abdominal anatomic anomalies were identified preoperatively, whereas no anatomic anomalies were detected within the chest. The patient had a right-sided stomach and spleen, liver at the midline, several vascular variations around the celiac axis, and intestinal malrotation, but the inferior vena cava and portal vein were normal. A pancreaticoduodenectomy was performed to treat the cancer. The postoperative course was favorable, and the patient was started on combined chemotherapy and radiotherapy 15 days after the surgery. In this case study, we report that pylorus preserving pancreaticoduodenectomy was successful for distal CBD cancer in a patient with rare situs anomalies.
Axis, Cervical Vertebra ; Common Bile Duct ; Drug Therapy ; Humans ; Jaundice ; Levocardia* ; Liver ; Lymphatic Diseases ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pancreaticoduodenectomy* ; Portal Vein ; Pylorus ; Radiotherapy ; Spleen* ; Stomach ; Thorax ; Vena Cava, Inferior

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Benign biliary strictures (BBSs) have been endoscopically managed with plastic stent placement. However, data regarding fully covered self-expandable metal stents (FCSEMSs) in BBS patients remain scarce in Korea. METHODS: Forty-one patients (21 men, 65.9%) with BBSs underwent FCSEMS placement between February 2007 and July 2010 in Asan Medical Center. Efficacy and safety were evaluated retrospectively. Patients were considered to have resolution if they showed evidence of stricture resolution on cholangiography and if an inflated retrieval balloon easily passed through the strictures at FCSEMS removal. RESULTS: The mean FCSEMS placement time was 3.2 (1.9-6.2) months. Patients were followed for a mean of 10.2 (1.0-32.0) months after FCSEMS removal. The BBS resolution rate was confirmed in 38 of 41 (92.7%) patients who underwent FCSEMS removal. After FCSEMS removal, 6 of 38 (15.8%) patients experienced symptomatic recurrent stricture and repeat stenting was performed. When a breakdown by etiology of stricture was performed, 14 of 15 (93.3%) patients with chronic pancreatitis, 17 of 19 (89.5%) with gall stone-related disease, 4 of 4 (100%) with surgical procedures, and 2 of 2 (100%) with BBSs of other etiology had resolution at FCSEMS removal. Complications related to stent therapy occurred in 12 (29%) patients, including post-ERCP pancreatitis (n=4), proximal migration (n=3), distal migration (n=3), and occlusion (n=2). CONCLUSIONS: Temporary FCSEMS placement in BBS patients offers a potential alternative to plastic stenting. However, because of the significant complications and modest resolution rates, the potential benefits and risks should be evaluated in further investigations.
Adult ; Aged ; Bile Duct Diseases/*therapy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/etiology ; Constriction, Pathologic ; Female ; Gallstones/etiology ; Humans ; Male ; Middle Aged ; Pancreatitis/etiology ; Retrospective Studies ; *Stents/adverse effects ; Time Factors

Percutaneous access to the surgical bed after pancreaticoduodenectomy can be a challenge, due to the post-operative anatomy alteration. However, immediate complications, such as surgical bed abscess or suspected tumor recurrence, are often best accessed percutaneously, as open surgical or endoscopic approaches are often difficult, if not impossible. We, hereby, describe a safe approach that is highly replicable, in accessing the surgical bed for percutaneous intervention, following pancreaticoduodenectomy.
Abscess/radiography/therapy ; Bile Duct Neoplasms/pathology/radiography ; Biopsy/methods ; Catheterization/*methods ; Cholangiocarcinoma/pathology/radiography ; Drainage/instrumentation/*methods ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local/pathology/radiography ; Pancreatic Diseases/radiography/therapy ; *Pancreaticoduodenectomy ; Postoperative Complications/radiography/*therapy ; Radiography, Interventional/methods ; Retroperitoneal Space

OBJECTIVETo investigate the pathogenesis of ischemic-type biliary lesions (ITBLs) in post-liver transplant patients and the possible therapeutic mechanisms of sirolimus.

METHODSThe clinic data of 32 post-liver transplant patients with ITBLs from May 2004 to December 2010 was analyzed. There were including 25 male and 7 female patients with a median age of 46 years (ranging from 19 to 61 years). Patients were divided into those who received sirolimus (sirolimus group) and those who did not (control group). The expression of IL-2, FoxP3, and IL-10 in the portal area, liver function indexes, and bile duct injury score were assessed pre-ITBL, when ITBLs were identified, and after 6 months of sirolimus treatment.

RESULTSCompared with pre-ITBL optical density (OD) values, there was a significantly increase in IL-2 OD(0.138 ± 0.050 in control group and 0.141 ± 0.052 in sirolimus group), but not FoxP3 and IL-10 OD in both groups at the time ITBLs were diagnosed. After 6 months of treatment, the IL-2, FoxP3, and IL-10 OD values in the control group were not different from those when ITBLs were diagnosed. There was a significant reduction in post-therapy IL-2 OD(0.107 ± 0.043, t = 2.087, P = 0.044), and a significant elevation in FoxP3(0.213 ± 0.039) and IL-10 OD(0.187 ± 0.048) in sirolimus group as compared with those when ITBLs were diagnosed(t = -3.822 and -4.350, both P < 0.01). There was a significant increase in serum levels of ALT, AST, total bilirubin, γ-glutamyl transpeptidase and ALP at the time ITBLs were diagnosed compared with pre-ITBL levels in both groups. After 6 months of treatment, the above indexes had not changed in the control group, but significantly improved in the sirolimus group, and the bile duct injury score in the sirolimus group had significantly decreased(4.4 ± 2.4, Z = -2.568, P = 0.010). The 1-year and 3-year graft survival rates in the control group were 6/13 and 5/13, respectively, and 17/19 and 13/19, respectively, in the sirolimus group (χ(2) = 7.166, P = 0.007; χ(2) = 5.398, P = 0.020, respectively).

CONCLUSIONSSirolimus can downregulate IL-2 expression and upregulate FoxP3 and IL-10 expression, thereby stimulating FoxP3+ Treg cells, suppressing immunopathological damage, and promoting epithelial repair in bile ducts.

Adult ; Bile Duct Diseases ; drug therapy ; Female ; Forkhead Transcription Factors ; metabolism ; Gene Expression Regulation ; drug effects ; Humans ; Interleukin-10 ; metabolism ; Interleukin-2 ; metabolism ; Ischemia ; diet therapy ; Liver Transplantation ; Male ; Middle Aged ; Postoperative Complications ; drug therapy ; Sirolimus ; therapeutic use ; Young Adult

Biliary cast describes the presence of casts within the biliary tree. It is resultant sequel of cholangitis and hepatocyte damage secondary to bile stasis and bile duct injury. Biliary cast syndrome was first reported in patient undergone liver transplantation. The pathogenesis of biliary cast is not clearly identified, but proposed etiologic factors include post-transplant bile duct damage, ischemia, biliary infection, or post-operative biliary drainage tube. Although biliary casts are uncommon, most of biliary cast syndrome are reported in the liver transplant or hepatic surgery patients. A few reports have been published about non-transplant or non-liver surgery biliary cast. We report two cases of biliary cast syndrome in non-liver surgery patients.
Acute Disease ; Ascariasis/diagnosis ; Bile Duct Diseases/*diagnosis/ultrasonography ; Bile Ducts/ultrasonography ; Cholagogues and Choleretics/therapeutic use ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects ; Female ; Gallstones/diagnosis ; Humans ; Liver Cirrhosis, Biliary/diagnosis/drug therapy ; Male ; Middle Aged ; Pancreatitis/etiology ; Tomography, X-Ray Computed ; Ursodeoxycholic Acid/therapeutic use

BACKGROUND/AIMS: Periampullary diverticulum (PAD) causes difficulty in the extraction of common bile duct (CBD) stones with conventional endoscopic therapy. Our study was designed to evaluate the effect of PAD on endoscopic large balloon dilation (EPLBD) with/without limited endoscopic sphincterotomy (EST) for CBD stone treatment. METHODS: We retrospectively reviewed cases of 141 patients treated CBD stones by EPLBD with/without limited EST at Gachon Gil Medical Center from September 2008 to February 2010. PAD were classified into three groups according to the location of the papilla and diverticulum. Clinical parameters, endoscopic parameters, and procedure outcomes were analyzed. RESULTS: PAD were identified in 46.1% (65/141), with 23 male (35.4%) and 42 female (64.6%) and a mean age of 72.9+/-11.1 years. Mean diameter of the stones was 14.8+/-6.0 mm and mean diameter of CBD was 21.6+/-7.7 mm. PAD group was significantly older than control group (72.9 vs. 68.6, p=0.043) and the incidence of large stone (> or =15 mm) was higher in PAD group (60.0% vs. 42.1%, p=0.034). Success rate of complete removal of stones in the first session was 32/65 patients (49.2%) and overall successful complete stone removal rates was 63/65 (96.9%). There was no significant difference between the PAD and control groups in success rate. Major complications were similar between two groups. CONCLUSIONS: PAD is associated with an increased incidence of large bile duct stones and older age. PAD seems to not increase technical failure rate or complication risk on EPLBD with/without limited EST.
Age Factors ; Aged ; Aged, 80 and over ; *Balloon Dilation ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct/anatomy & histology ; Diverticulum/*diagnosis ; Duodenal Diseases/*diagnosis ; Female ; Gallstones/surgery/*therapy ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome

No abstract available.
Autoimmune Diseases/*diagnosis/drug therapy/immunology ; Azathioprine/therapeutic use ; Common Bile Duct/pathology ; Emphysema ; Fibrosis ; Humans ; Immunoglobulin G/blood ; Immunosuppressive Agents/therapeutic use ; Lung/*radiography ; Male ; Middle Aged ; Pancreatitis/*diagnosis/drug therapy/immunology ; Stents ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Endoscopic retrograde cholangiopancreatograpy (ERCP) is often used for the diagnosis and treatment of pancreaticobiliary diseases in the elderly patients. However, few studies have assessed its efficacy and safety in the very elderly. The purpose of this study was to evaluate the clinical outcomes of ERCP in the very elderly patients. METHODS: Eight hundreds two patients who underwent ERCP at Seoul National University Bundang hospital were enrolled retrospectively. They were divided into three groups according to their ages (non-elderly group, elderly group and very-elderly group; <65, 65-79 and > or =80, respectively). The indications and clinical outcomes including the complications of ERCP were compared among groups. RESULTS: The most common indication of ERCP was acute cholangitis in all the three groups. Periampullary diverticulum was more frequently observed in elderly and very-elderly patients than in younger patients. Mean duration of hospitalization was not different among three groups. ERCP success rate in all enrolled patients was approximately 90%, and there was no difference in terms of technical success rate between groups (p=0.1). However, the number of ERCP sessions was significantly higher in the very-elderly patients compared to in the non-elderly and elderly (1.38 vs. 1.13 and 1.18 respectively; p<0.001). There was no difference in mortality and complication rate between groups. CONCLUSIONS: ERCP can be performed safely in very-elderly patients. Therefore, only age should not be regarded as one of the major determining factors whether to perform ERCP.
Acute Disease ; Age Factors ; Aged ; Aged, 80 and over ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects/*methods ; Cholangitis/diagnosis ; Common Bile Duct Diseases/diagnosis ; Diverticulum/diagnosis ; Female ; Humans ; Length of Stay ; Male ; Middle Aged ; Pancreatic Diseases/*diagnosis/therapy ; Retrospective Studies